In a report by the American College of Cardiology/American Heart Association (AHA/ACC), writing committee members provided an evidence-based update on the evaluation, management, and monitoring recommendations for adults with congenital heart disease.
To optimize outcomes in complex congenital heart disease, the AHA/ACC writing committee emphasized the importance of using an integrated multidisciplinary approach to care. Gaps in congenital heart disease adult care, such as lack of appropriate follow-up and difficulty finding specialized providers, are barriers that prevent optimized outcomes in these patients.
In their report, the committee recommends several tools to evaluate and manage the treatment course of adult patients with congenital heart disease, including:
(class of recommendation I; level of evidence C, expert opinion)
(class of recommendation I; level of evidence B, nonrandomized)
- Cardiac magnetic resonance imaging
(class of recommendation I; level of evidence B, nonrandomized, particularly in patients who are at risk for right ventricular enlargement and dysfunction)
- Cardiac computed tomography
(class of recommendation IIa; level of evidence C, limited data)
- Cardiac catheterization
(class of recommendation I; level of evidence C, limited data, particularly in patients with adult congenital heart disease classification II and III)
- Exercise Testing
(class of recommendation IIa; level of evidence B, nonrandomized)
Additionally, the AHA/ACC provided evidenced-based treatment recommendations for specific lesions, including shunt, left-sided obstructive, right-sided, and complex lesions. For shunt lesions characterized as atrial septal defects, for instance, pulse oximetry is recommended to determine the direction and magnitude of shunt as well as to guide clinicians in developing the most appropriate treatment option. For valvular pulmonary stenosis in right-sided lesions, the writing committee recommends cyanosis from interatrial right-to-left communication and/or exercise intolerance or balloon valvuloplasty (class of recommendation I; level of evidence B, nonrandomized).
Transition education is also recommended for adolescent and young patients with congenital heart disease to improve their ability to navigate the healthcare system as adults. With regard to physical activity, the writing committee recommends periodic assessment of patients’ activity level to develop an exercise program that features intensities tailored to the patient’s individual clinical status.
Cardiac rehabilitation is also suggested as a means of increasing exercise capacity in adult patients with congenital heart disease (class of recommendation IIa; level of evidence B, nonrandomized). Depression and anxiety as well as other mental health issues should also be monitored on a regular basis, according to the writing committee (class of recommendation I; level of evidence B, nonrandomized).
In addition to the adult congenital heart disease evaluation and treatment recommendations, the AHA/ACC indicated that there is a greater need for comprehensive multicenter and population databases and registries to address common clinical questions regarding the condition. “Novel study methodologies are needed to ascertain effectiveness of diagnostic and therapeutic options when each disease is sufficiently rare and events occur over sufficiently long periods that [randomized controlled trials] are impractical,” the committee wrote.
Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines [published online August 16, 2018]. J Am Coll Cardiol. doi: